• Yi-Hsin Hung, An-Li Yu, Yi-Chieh Chen, Cheng-Hsuan Tsai, Mao-Yuan Su, Chia-Tung Shun, Hsueh-Wen Hsueh, Jyh-Ming JuangJimmyJDepartment of Internal Medicine, Division of Cardiology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan., Ming-Jen Lee, Ping-Huei Tseng, Chia-Hua Hsu, Sung-Tsang Hsieh, Chi-Lun Ko, Kon-Ping Lin, Wen-Chung Yu, Mei-Fang Cheng, Chi-Chao Chao, and Yen-Hung Lin.
• Department of Internal Medicine, Division of Cardiology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan.
• J Formos Med Assoc. 2024 Oct 10.

BackgroundRNA interference therapeutics reduce transthyretin production; however, their effect on hereditary transthyretin amyloid cardiomyopathy (ATTR-CA) remains unclear. We aimed to investigate alterations in technetium-99 m (99mTc)-pyrophosphate (PYP) single-photon emission computed tomography/computed tomography (SPECT/CT) outcomes in patients receiving patisiran or vutrisiran.MethodsWe retrospectively identified individuals with hereditary ATTR-CA who received patisiran or vutrisiran. First and second 99mTc-PYP SPECT/CT data, including visual grading, planar heart to contralateral lung (H/CL) ratio, and volumetric heart to lung (H/L) ratio were assessed.ResultsEight patients with hereditary ATTR-CA were enrolled. Cohort A included four patients who underwent their first 99mTc-PYP SPECT/CT imaging at the initiation of small interfering RNA (siRNA) treatment, while cohort B comprised four patients who had been receiving siRNA treatment before their first 99mTc-PYP SPECT/CT imaging (median duration 1281 days). Overall, there were numerical reductions in planar H/CL ratio (1.7 ± 0.2 to 1.6 ± 0.1, p = 0.050) and a significant improvement in volumetric H/L ratio (4.0 ± 0.9 to 3.5 ± 0.4, p = 0.035). Although without significance, subgroup analysis showed more pronounced changes in cohort A for both planar H/CL ratio and volumetric H/L ratio (-20.1 ± 12.6% and -17.1 ± 11.4%) compared to cohort B (-3.3 ± 11.2% and -4.3 ± 12.7%).ConclusionOur results demonstrated a significant decrease in volumetric H/L ratio in hereditary ATTR-CA patients receiving RNA interference therapeutics.Copyright © 2024 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.

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